Buried bumper syndrome in percutaneous endoscopic gastrostomy with a jejunal extension tube in patients undergoing levodopa-carbidopa intestinal gel treatment.

BACKGROUND: Levodopa-carbidopa intestinal gel (LCIG) treatment is an effective Parkinson's disease (PD) treatment that requires percutaneous endoscopic gastrostomy with a jejunal extension tube (PEG-J). Buried bumper syndrome (BBS) is an uncommon but significant complication of PEG-J for LCIG. Case presentation A 71-year-old man had been undergoing LCIG therapy for PD since a PEG-J was implemented at our department two years previously. He presented with appetite loss. Computed tomography showed that the gastrostomy bumper was buried in the gastric wall. The patient was surgically treated with the simultaneous removal and replacement of PEG-J. Postoperative gastrocutaneous fistula occurred, which was conservatively treated. CONCLUSIONS: Notably, patients and medical staff should be aware that patients with PD on LCIG treatment have a high risk of BBS in PEG-J and that there might be some patients with latent BBS. When simultaneous removal and replacement surgery is performed, establishing a new route at the stomach and abdominal wall is recommended.

Laparoscopic Heller myotomy with Dor fundoplication for esophageal achalasia treatment after distal gastrectomy and Billroth-II reconstruction.

Laparoscopic Heller myotomy with Dor fundoplication is the standard surgical treatment for esophageal achalasia. However, there are few reports on the use of this method after gastric surgery. We report a case of a 78-year-old man who underwent laparoscopic Heller myotomy with Dor fundoplication for achalasia after distal gastrectomy and Billroth-II reconstruction. After the intraabdominal adhesion was sharply dissected using an ultrasonic coagulation incision device (UCID), Heller myotomy was performed 5 cm above and 2 cm below the esophagogastric junction using the UCID. To prevent postoperative gastroesophageal reflux (GER), Dor fundoplication was performed without cutting the short gastric artery and vein. The postoperative course was uneventful, and the patient is in good health without symptoms of dysphagia or GER. Although per-oral endoscopic myotomy is becoming the mainstay of treatment for achalasia after gastric surgery, laparoscopic Heller myotomy with Dor fundoplication is also an effective strategy.

Segmental cervical esophagectomy with free jejunal flap reconstruction for cervical esophageal cancer in patients with previous history of gastric surgery: a report of two cases.

Although free-flap jejunal reconstruction is frequently performed after cervical esophagectomy for cervical esophageal cancer, the procedure after gastric surgery has not been reported. We encountered two patients with esophageal cancer and previous gastric surgeries who eventually underwent segmental esophagectomy with free-flap jejunal reconstruction. Case one involved a 75-year-old man who underwent abdominal abscess and duodenal ulcer perforation surgeries (abdominal drainage and subsequent gastrojejunal bypass). A type 0-IIa tumor was located posterior to the cervical esophagus's right wall, 21 cm from the incisor, without lymph node swelling or distant metastasis. The left lobe of the thyroid gland was mobilized to ensure an oral resection margin. Severe abdominal adhesions required careful adhesiolysis to harvest the jejunum (20 cm long) 40 cm from the jejunojejunostomy. An end-to-side and side-to-end esophagojejunostomy were performed for the proximal and distal ends, respectively. Case two involved a 75-year-old male with a history of distal gastrectomy with Billroth I reconstruction for early gastric cancer. A submucosal tumor-like lesion was located on the cervical esophageal wall on the left side, 21 cm from the incisor. The distal esophagus required additional segmental resection because the anal resection line was close to the tumor. Jejunum (10 cm long) 30 cm from Ligament of Treitz was harvested. An end-to-side and end-to-end esophagojejunostomy for the proximal and distal ends, respectively, was performed. This surgery requires a thorough preoperative examination to ensure an adequate surgical margin and a careful free-flap harvest based on post-gastric surgery anatomy.

Therapeutic strategy aiming at R0 resection for borderline-resectable esophageal squamous cell carcinoma using induction chemotherapy with docetaxel, cisplatin, and 5-fluorouracil.

OBJECTIVE: Treatment for borderline resectable (cT3br) esophageal squamous cell carcinoma (SCC) is currently undefined. This study aimed to analyze the outcome of treatment strategies including induction chemotherapy with docetaxel, cisplatin, and 5-fluorouracil (DCF) against T3br esophageal SCC. METHODS: A total of 32 patients with cT3br esophageal SCC enrolled in this study were treated with two cycles of DCF induction therapy. RESULTS: The overall response rate to DCF induction therapy was 62.5%, while the disease control rate was 93.8% (complete response (CR), three; partial response (PR), 17; stable disease (SD), 10; progressive disease (PD), 2). After DCF induction chemotherapy, 27 patients underwent conversion surgery (CS) and five patients underwent definitive chemoradiotherapy (CRT). Out of 27 patients who underwent CS, 17 underwent transthoracic esophagectomy and 10 underwent thoracoscopic esophagectomy. Anastomotic leakage occurred in five patients (18.5%) and pneumonia in four (14.8%). Recurrent laryngeal nerve paralysis and arrhythmia were observed in two patients (7.4%). The R0 resection rate was 81.5%. Among the five patients who underwent definitive CRT, only one patient (20.0%) achieved CR. Two patients (40.0%) had PR and two (40.0%) had PD. Salvage esophagectomy was performed in one patient after definitive CRT. The 1-, 3-, and 5-year overall survival rates were 75.0, 50.6, and 46.4%, respectively, whereas the 1-, 3-, and 5-year disease-free survival rates were 54.9, 38.8, and 38.8%, respectively. CONCLUSION: DCF induction therapy and subsequent CS or definitive CRT are promising treatment strategies for cT3br esophageal SCC.

Successful combined laparoscopic and thoracoscopic surgery for esophago-mediastinal fistulae.

Minimally invasive surgeries have been developed, not only for gastrointestinal cancer, but also for benign or emergency cases. We report the case of a 62-year-old male who underwent laparoscopic and thoracoscopic combined surgery for an esophago-mediastinal fistula caused by a press-through package. In the initial laparoscopic phase, transhiatal dissection of the lower thoracic esophagus and harvesting of the greater omentum were performed. In the thoracoscopic phase, resection of the fistula and esophageal wall closure were performed. Thereafter, the greater omentum was lifted via the esophageal hiatus and wrapped around the repaired part of the esophagus for reinforcement. The total operative time was 371 min, with 163 and 208 min for the laparoscopic and thoracoscopic phases, respectively. In total, 20 ml of blood was lost. No perioperative complications or recurrences were observed. Laparoscopic and thoracoscopic combined omentoplasty was effective for refractory esophago-mediastinal fistula.

Proton Beam Therapy With Space-Making Surgery (Omental Plombage) for Oligorecurrent Liver Metastasis of Esophageal Adenocarcinoma.

Proton beam therapy (PBT) with space-making surgery has been used recently; however, its effectiveness for recurrent esophageal cancer (EC) is unclear. We herein report an unusual case of successful PBT with space-making surgery (omental plombage) for recurrent liver metastasis after EC surgery. A 58-year-old Japanese man underwent proximal gastrectomy for esophagogastric (EG) junction cancer seven months before presentation to our hospital. Microscopic findings after the surgery showed that the tumor was adenocarcinoma of the EG junction (pT1N0M0, stage I). Seven months after the proximal gastrectomy, liver metastases in S6 and S8 were revealed by positron emission tomography-computed tomography. Initial PBT was performed for those two liver metastases, and complete response (CR) was obtained for both liver metastases. Recurrence of liver metastasis in S2 was found eight months after the first PBT, and CR was achieved by chemotherapy. However, new liver metastasis recurred in S2. Considering the effects of radiation exposure on the surrounding gastrointestinal organs, we performed space-making surgery to place the omentum around the liver metastasis. We were able to complete the second PBT for the liver metastasis with 72.6 Gy relative biological effectiveness in 22 fractions. After the second PBT, the patient survived for seven years without recurrence. PBT with space-making surgery (omental plombage) for recurrent liver metastasis after EC surgery is considered to be a therapeutic option.

Strategy Treatment of cT4b Esophageal Squamous Cell Carcinoma Using Docetaxel, Cisplatin, and 5-Fluorouracil.

BACKGROUND/AIM: This study analyzed the outcomes of docetaxel, cisplatin, and 5-fluorouracil (DCF) therapy and DCF plus concurrent radiotherapy (DCF-RT), both followed by conversion surgery, if possible, in patients with cT4b esophageal cancer. PATIENTS AND METHODS: Forty-six patients with cT4b esophageal cancer, including borderline cT4b lesions, were eligible. Borderline cT4b lesions were treated with induction DCF therapy. For definitive cT4b lesions, definitive DCF-RT was administered. Patients unsuitable for induction DCF therapy or DCF-RT were treated with other therapies. After treatment, conversion surgery (CS) was performed for the residual tumor in resectable cases. RESULTS: Induction DCF therapy was administered to 12 patients (group A), and DCF-RT was provided to 18 patients (group B). Meanwhile, other therapies were provided to 16 patients (group C). The 1-, 3-, and 5-year overall survival (OS) rates were 66.7, 30.0, and 15.0%, respectively, in group A; 66.7, 37.5, and 37.5%, respectively, in group B; and 62.5, 0, and 0%, respectively, in group C. DCF-RT tended to prolong survival, albeit without significance (p=0.1040). The group A + B had significantly better overall survival than group C (p=0.0437). Fourteen patients underwent CS (30.4%), and patients who underwent CS had significantly better overall survival than those who did not undergo surgery (p=0.0291). CONCLUSION: Induction DCF or DCF-RT is promising for the treatment of cT4b esophageal cancer. Effective CS including combined resection of the invaded organ can contribute to improved therapeutic outcomes.

Dislocation of the gastric conduit reconstructed via the posterior mediastinal route is a significant risk factor for anastomotic disorder after McKeown esophagectomy.

BACKGROUND: Anastomotic disorder of the reconstructed gastric conduit is a life-threating morbidity after thoracic esophagectomy. Although there are various reasons for anastomotic disorder, the present study focused on dislocation of the gastric conduit (DGC). METHODS: The study cohort comprised 149 patients who underwent transthoracic esophagectomy. The relationships between DGC and peri- and postoperative morbidities were analyzed retrospectively. Data were analyzed to determine whether body mass index (BMI) and extension of the gastric conduit were related to DGC. Uni- and multivariate Cox regression analyses were performed to identify the factors associated with anastomotic disorder. RESULTS: DGC was significantly related to anastomotic leakage (P < .001), anastomotic stricture (P = .018), and mediastinal abscess/empyema (P = .031). Compared with the DGC-negative group, the DGC-positive group had a significantly larger mean preoperative BMI (23.01 ± 3.26 kg/m2 vs. 21.22 ± 3.13 kg/m2, P = .001) and mean maximum cross-sectional area of the gastric conduit (1024.75 ± 550.43 mm2 vs. 619.46 ± 263.70 mm2, P < .001). Multivariate analysis revealed that DGC was an independent risk factor for anastomotic leakage (odds ratio: 4.840, 95% confidence interval: 1.770-13.30, P < .001). Body weight recovery tended to be better in the DGC-negative group than in the DGC-positive group, although this intergroup difference was not significant. CONCLUSION: DGC reconstructed via the posterior mediastinal route is a significant cause of critical morbidities related to anastomosis. In particular, care is required when performing gastric conduit reconstruction via the posterior mediastinal route in patients with a high BMI.

The Prognostic Significance of Plakophilin-1 Expression in Esophageal Cancer.

BACKGROUND/AIM: Plakophilin 1 (PKP1) expression is inversely related to cancer grade. This study aimed to evaluate whether PKP1 is a prognostic marker for esophageal cancer (EC). MATERIALS AND METHODS: We tested immunohistochemically for PKP1 in squamous cell carcinoma EC specimens from 99 patients, including cytoplasmic (C), membrane (M), and nuclear (N) cellular areas, and analyzed their relationships with clinicopathological factors. RESULTS: PKP1stains were stratified into strong and weak for all three cellular areas. Staining was inversely related to tumor depth (C: p=0.002, M: p=0.00007, N: p=0.02), lymph node metastasis (C: p=0.003, M: p=0.001, N: p=0.004) and pathological stage (C: p=0.0004, M: p=0.0001, N: p=0.006). Cytoplasmic and membrane staining were inversely related to vessel invasion. Patients with strong C stain had a better overall survival than those with weak C stains (p=0.01). Disease-free survival of patients with strong M stains was better than that of those with weak staining (p=0.01). CONCLUSION: Cytoplasmic and membrane PKP1 expression is a possible prognostic marker for EC.

A Pilot Study Analyzing the Clinical Utility of Comprehensive Genomic Profiling Using Plasma Cell-Free DNA for Solid Tumor Patients in Japan (PROFILE Study).

BACKGROUND: The clinical utility of plasma cell-free DNA in precision cancer medicine has not been established. A pilot study was conducted to investigate the clinical utility of comprehensive genomic profiling by liquid biopsy in a Japanese population. METHODS: In this PROFILE study, 102 patients with advanced solid tumors who showed progression with standard systemic therapy underwent liquid biopsy between August 2017 and February 2020. Liquid biopsy was performed using Guardant360. RESULTS: Of the 102 patients, 56 were women, and the median age was 65 years. Regarding the types of cancer, 31 were hepatobiliary and pancreatic cancer, 17 were gastrointestinal cancer, and 13 were breast cancer. Frequently altered genes were TP53 (53.9%, 46/102), KRAS (25.5%, 26/102), PIK3CA (19.6%, 20/102), and EGFR (17.6%, 18/102). At least one genetic aberration was detected in 92 patients (90.2%). Actionable mutation was discovered in 88 patients (86.3%), and 67 patients (65.7%) were clinical trial candidates. Of the 102 patients, 22 (21.6%) were able to receive biomarker-matched therapy. Their best responses were as follows: 1 complete response, 3 partial responses, 7 stable diseases, and 11 progressive diseases. Additionally, the treated patients were divided on the basis of matching scores (≥ 50% vs. < 50%). The patients were divided into high and low groups. The high group had a higher disease control rate (DCR) of 75% compared with 20% in the low group (P = 0.010). CONCLUSIONS: The results indicate that liquid biopsy is useful for identifying actionable mutations associated with the clinical response of selected patients.

Histopathological evaluation of the effectiveness of oral Eppikajutsuto treatment for lymphatic malformation.

BACKGROUND: Lymphatic malformation (LM) is a congenital disease caused by lymphatic vessel malformation. Although standard therapies for LMs are sclerotherapy and/or surgical excision, a new therapy using Japanese herbal medicine Eppikajutsuto (TJ-28) has been recently reported as clinically effective. We aimed to experimentally confirm the therapeutic effectiveness of TJ-28 for LMs. METHODS: LM lesions were generated in the mesentery and peritoneum of mice by intraperitoneal injection of Freund's incomplete adjuvant. Mice with LMs were treated by gavage or dietary administration of TJ-28 for 2 months. Formalin-fixed paraffin-embedded tissue sections of mesentery and peritoneum tissues were histologically and immunohistochemically examined by focusing on lymph nodes and perinodal lymph vessels. RESULTS: Multiple Freund's incomplete adjuvant-associated foreign-body granulomas were formed in the mesentery and peritoneum, resulting in congestion of lymph fluid and dilatation of lymph vessels. The numbers and sizes of lymph nodes were not significantly different between TJ-28-treated and control groups. However, the luminal areas of lymphatic vessels were reduced significantly in the TJ-28 treatment group by both gavage and dietary administrations. CONCLUSION: TJ-28 conspicuously reduced congestion of lymph fluid. This is the first histopathological evaluation of LM model mice to study the effectiveness of oral TJ-28 treatment.

Thoracic endovascular aortic repair for esophageal cancer invading the thoracic aorta: a questionnaire survey study.

BACKGROUND: Locally advanced esophageal cancer occasionally invades the aorta, and hemorrhage from the esophagoaortic fistula can cause sudden death. Thoracic endovascular aortic repair (TEVAR) enables hemostasis in such cases, and prophylactic TEVAR can prevent fatal hemorrhagic events during treatment. However, its efficacy in Japan has not been evaluated. This study aimed to clarify the clinical significance of TEVAR in esophageal cancer patients. METHODS: The Japan Esophageal Society conducted a questionnaire survey targeting authorized or semi-authorized member institutes of the Authorized Institutes for Board Certified Esophageal Surgeons. Patients who underwent TEVAR for esophageal cancer were identified from 19 institutes. Data on patient demographics, treatment performed, and survival rate were obtained using the questionnaire. The Kaplan-Meier method was used for survival analysis and to compare differences in survival rates between those who underwent TEVAR for hemorrhage and those for preoperative prophylaxis. RESULTS: Of the 41 patients identified, 20 patients underwent TEVAR for hemorrhage or impending hemorrhage from the esophagoaortic fistula, while 21 patients underwent TEVAR as preoperative prophylaxis. The median survival time after TEVAR was 135 days in the hemorrhage or impending hemorrhage group and 378 days in the preoperative prophylaxis group. Eighteen patients underwent esophagectomy after TEVAR. No hemorrhagic event was observed during the perioperative period. The median survival time of the patients who underwent esophagectomy was 373 days. Some patients who achieved R0 resection obtained long-term survival. CONCLUSION: TEVAR is an efficacious modality to control a life-threatening hemorrhage from esophagoaortic fistula and helps to prolong the survival of patients with locally advanced esophageal cancer invading the aorta.

Treatment strategy for brain metastases from esophageal cancer.

BACKGROUND: This study aimed to examine the treatment outcomes of patients with brain metastases from esophageal cancer. Brain metastases from esophageal cancer are rare and have a poorer prognosis than brain metastases from lung and breast cancer. METHODS: This study included patients who were diagnosed with and treated for esophageal cancer in our department and subsequently developed brain metastases between April 2010 and December 2014. We examined the differences in survival in patients based on receiving chemotherapy. RESULTS: In total, 8 patients (7 men and 1 woman) with a mean age of 65 years (range 51-73) were included. Seven presented with neurologic symptoms. Two were diagnosed via computed tomography (CT), 5 via magnetic resonance imaging, and 1 via positron emission tomography/CT. They were treated using whole-brain irradiation or with a gamma knife. In 5 patients, chemotherapy was administered after treatment of the brain metastases. The mean survival from the start of treatment was 358 days (range 31-1196). CONCLUSION: The relatively successful local control of brain metastases in these patients indicates that long-term survival may be attainable via concomitant chemotherapy.

Nutritional status predicts adjuvant chemotherapy outcomes for stage III colorectal cancer.

OBJECTIVES: Previously, adjuvant chemotherapy using oxaliplatin was a standard treatment for patients with node-positive colorectal cancer (CRC) who underwent curative surgery. The factor predicting adverse events and therapeutic effect have not yet been established. METHODS: A retrospective cohort of 42 patients diagnosed with stage III CRC between April 2009 and March 2013 in our institution were included in this study. The indicators of host nutritional status were body weight (BW), body mass index (BMI), serum albumin, Onodera's prognostic nutritional index (OPNI), and Glasgow Prognostic Score (GPS). The indicators of host immunocompetence was total lymphocyte counts, total neutrophil counts, granulocytes/lymphocytes ratio (G/L ratio). RESULTS: The overall recurrence rate was 26.1%. Patients who had a recurrence were more likely to be older. The recurrence was not associated with type of regimen or adverse events. The cases with a few cumulative doses and relative dose intensity of oxaliplatin experienced significantly more recurrence. Nutritional status indicators, such as the serum albumin level, OPNI, and the modified Glasgow prognostic score (mGPS) were associated with the adjuvant chemotherapy outcome. Our study results indicated worse nutritional status induced worse disease-free survival (DFS) and more recurrence. CONCLUSION: The host's nutritional status associated with outcomes in stage III CRC patients.

[A Case of Advanced Gastric Cancer with Multiple Bone Metastases, Virchow Lymph Node and Para-Aortic Lymph Node Metastases That Responded to Combined Modality Therapy and Underwent Conversion Surgery].

A 41-year-old woman with type 3 advanced gastric cancer and Virchow lymph node, para-aortic lymph node, and multiple bone metastases was diagnosed with U-less cType 3 cT4aN3M1, cStage IV. We administered docetaxel, cisplatin, and S-1 (DCS)therapy for unresectable gastric cancer. After 11 courses of DCS, we confirmed that the distant lymph node metasta- ses were significantly reduced. We performed radiotherapy(30 Gy/10 Fr)on the thoracic lumber vertebrae. Because the patient was successfully downstaged, we performed total gastrectomy with Roux-en-Y reconstruction. The histopathological diagnosis was ypT3N2M0, ypStage III A. In this case, DCS therapy successfully treated gastric cancer with distant metastases, including multiple bone metastases.

Salvage esophagectomy combined with partial aortic wall resection following thoracic endovascular aortic repair.

OBJECTIVE: Definitive chemoradiotherapy is useful for locally advanced esophageal cancer. However, salvage esophagectomy is required when residual or recurrent tumor is evident after chemoradiotherapy. We performed salvage esophagectomy combined with partial aortic wall resection after thoracic endovascular aortic repair for aortic invasion, and we evaluated the results. METHODS: Four patients underwent esophagectomy combined with aortic wall resection after thoracic endovascular aortic repair because the lesion was diagnosed as stage T4b. We evaluated short-term outcomes, including duration of thoracic surgery, blood loss, duration of intubation, intensive care unit stay, and postoperative morbidity, as well as survival after salvage surgery. RESULTS: Lesions were resected with no intraoperative procedural adverse events. Mean thoracic operation time was 444 min, and mean thoracic blood loss was 506 g. In all patients, complete resection of the lesion was possible with no intraoperative adverse events. All patients were extubated on postoperative day 0, and all were discharged from the intensive care unit on postoperative day 1. One patient experienced grade II wound pain (bilateral chest pain), and another patient experienced difficult sputum expectoration (grade IIIA). The mean follow-up time was 19.8 months, and two patients were alive with no disease recurrence at the time of this report. However, the remaining two patients had died secondary to lymph node metastasis. CONCLUSIONS: Salvage esophagectomy combined with partial aortic wall resection after thoracic endovascular aortic repair provides acceptable short-term outcomes. Future studies are needed to evaluate long-term survival and patient selection criteria.

Concurrent Chemoradiotherapy with Docetaxel, Cisplatin, and 5-fluorouracil Improves Survival of Patients with Advanced Esophageal Cancer Compared with Conventional Concurrent Chemoradiotherapy with Cisplatin and 5-fluorouracil.

Purpose: To compare treatment outcomes and adverse events between concurrent chemoradiotherapy with docetaxel, cisplatin, and 5-fluorouracil (DCF-RT) and conventional concurrent chemoradiotherapy with cisplatin and 5-fluorouracil (CF-RT). Methods and Materials: We retrospectively investigated treatment outcomes and adverse events in 121 patients with advanced esophageal cancer who underwent concurrent chemoradiotherapy with CF-RT (n = 83) or DCF-RT (n = 38). In the CF-RT group, patients were administered cisplatin (70 mg/m2) and 5-fluorouracil (700 mg/m2) for 5 days; in the DCF-RT group, patients were administered docetaxel (50 mg/m2), cisplatin (50 mg/m2), and 5-fluorouracil (500 mg/m2) for 5 days. The radiotherapy dose was 1.8-2 Gy per session, up to a total of 50-60 Gy. Results: The complete response (CR) rate was 37.8% in the CF-RT group and 52.6% in the DCF-RT group. Overall survival (OS) rates at 2 and 3 years were 45.0% and 37.5%, respectively, in the CF-RT group and 62.9% and 56.7%, respectively, in the DCF-RT group, with a significant intergroup difference (p = 0.032). Progression-free survival rates at 2 and 3 years were 44.1% and 36.9%, respectively, in the CF-RT group and 45.0% and 45.0%, respectively, in the DCF-RT group (p = 0.10). Local control rates at 2 and 3 years were 59.1% and 54.6%, respectively, in the CF-RT group and 71.8% and 71.8%, respectively, in the DCF-RT group (p = 0.12). The incidence of Grade 3/4 leukopenia was 55.4% (n = 46) in the CF-RT group and 78.9% (n = 30) in the DCF-RT group, with a significant intergroup difference (p = 0.022). The incidence of Grade 3/4 neutropenia was 47.0% (n = 39) in the CF-RT group and 65.8% (n = 25) in the DCF-RT group, with a notable albeit not statistically significant difference between the groups (p = 0.054). There were no significant intergroup differences in anemia, thrombocytopenia, radiation-induced dermatitis, radiation esophagitis, or late adverse events. Conclusions: Rates of OS and CR were improved after treatment with DCF-RT compared with CF-RT. Although DCF-RT-treated patients had higher rates of leukopenia, treatment safety was ensured through proper management of myelotoxicity. DCF-RT is a promising treatment regimen for advanced esophageal cancer.

Waardenburg syndrome with isolated deficiency of myenteric ganglion cells at the sigmoid colon and rectum.

Waardenburg syndrome (WS) has the characteristic clinical features caused by the embryologic abnormality of neural crest cells. WS patients sometimes suffer from functional intestinal obstruction. When it is Hirschsprung disease (HD), the WS is diagnosed as type 4 WS. We report a case of WS which did not have myenteric ganglion cells in the sigmoid colon and rectum. Whether to diagnosis this case as type 1 or 4 WS is controversial. Moreover, this is the third report which has peristalsis failure caused by abnormal myenteric plexus. In all three cases, the eosinophils had aggregated in the myenteric layer of the transition zone. During embryonic life, enteric ganglion cells migrate to the myenteric layer from the proximal to the distal side sequentially and, subsequently, to the submucosal layer through the circular muscle. Therefore, we hypothesize that myenteric ganglion cells that had already migrated were eliminated by an eosinophil-mediated mechanism in these three cases. We believe this report may be helpful to elucidate the pathogenesis of some types of HD.